Contemporary Management of Severe Carcinoid Heart Disease

Carcinoid heart disease (CHD), despite its rarity, is a serious complication of carcinoid syndrome (CS). Approximately 50% of patients with CS will develop CHD during the course of their disease. It predominantly affects the right-sided heart valves, leading to valve defect and right heart failure. In patients with advanced functional neuroendocrine tumors, diagnosing and managing CHD remains a challenge.

A 71-year-old male with a metastatic midgut neuroendocrine tumor and CS presented with worsening right heart failure. Despite initiating treatment appropriately, he deteriorated and ultimately died, highlighting the difficulty of CHD management in advanced cases.

Early detection and prompt management of CS are vital to prevent the progression to CHD. Therefore, a carefully planned sequential management approach should be developed for a generalized adaptation. Multidisciplinary care is vital, and new percutaneous interventions could provide promising options for high-risk patients.

Routine screening of CHD in NET patients, timely intervention, and specialized multidisciplinary management are vital for improving outcomes in CHD.